CTEPH | Pulmonology & Critical Care | Loyola Medicine

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Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

Overview and Facts about Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

Chronic thromboembolic pulmonary hypertension (CTEPH) is a form of high blood pressure that affects the lungs. It's usually caused by scar tissue left behind by blood clots. In some cases, CTEPH may also be linked to inflammatory diseases or cancer.

Anyone can develop CTEPH, but people who have had blood clots in their lungs are at an especially high risk. About 75% of people with CTEPH have a pulmonary embolism or a blood clot in the lungs.

Signs and Symptoms of Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

Symptoms of CTEPH can include the following:

  • Shortness of breath
  • Fatigue
  • Palpitations
  • Chest heaviness
  • Dizziness
  • Fainting
  • Leg swelling

The early symptoms of CTEPH are usually mild, though they may worsen with exercise. Symptoms may become more severe over time.

Causes and Risk Factors of Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

CTEPH is caused by blood clots in the lungs, known as pulmonary embolisms (PEs). These blood clots leave behind scar tissue, which narrows the arteries in the lungs.

Risk factors for PEs include:

  • Being overweight
  • Being pregnant
  • Smoking
  • Taking certain hormone medications
  • A history of heart disease

Other risk factors for CTEPH may include:

  • A history of large PEs
  • Thyroid disorders
  • Blood clotting disorders
  • Having a certain blood type

If you have a history of blood clots, speak to your doctor to learn more about your risk for CTEPH. Report any new or worsening symptoms to a medical provider right away.

Tests and Diagnosis of Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

CTEPH can be difficult to diagnose. The early symptoms of the disease are often vague and may be confused with other conditions. If your doctor believes you may have CTEPH, he or she may order several different tests, such as:

  • Lung ventilation-perfusion scans
  • Right-heart catheterization
  • Echocardiograms
  • Angiograms
  • CT scans
  • Pulmonary function tests

Imaging tests like CT scans can help your doctor examine your heart and lungs. Some imaging tests involve an injected contrast dye.

These tests can also reveal blockages or scarring in the arteries. Other tests, like pulmonary function tests, can show how well your lungs are working.

Your doctor may also order laboratory tests to check your blood. These tests can help rule out other conditions with similar symptoms.

Treatment and Care for Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

CTEPH can be a serious condition. If you're diagnosed with CTEPH, your doctor may refer you to a pulmonary and critical care specialist. These doctors specialize in treating life-threatening conditions that affect your heart and lungs.

In most cases, the recommended treatment for CTEPH is pulmonary thromboendarterectomy (PTE). During this surgery, the patient's heart is stopped, and they are put on a heart-lung machine. The surgeon then removes the clots from the patient's arteries.

PTE has a 90 percent success rate, but it's a very invasive surgery and may not be right for everyone. If you are unable to undergo PTE, your doctor may recommend balloon pulmonary angioplasty.

This procedure can help destroy clots in the pulmonary arteries. Your doctor may also suggest medication to help control your blood pressure.